We describe a 60-year old man with the history of radical correction of the Tetralogy of Fallot (TOF) in the year 1964. This patient has had a long lasting decompensation of a severe right heart failure with ascites and pulmonary hypertension. On echocardiography he had residual mild pulmonary stenosis (PS) and severe pulmonary and tricuspid regurgitation (TR), moderate aortic and mitral regurgitation. He also had residual ventricular septal defect (VSD) and severe pulmonary hypertension with the maximal gradient on TR 83 mmHg. He was considered unoperable by his cardiologist, however patient decided to undergo a high-risk operation. The operation comprised pulmonary and aortic valve replacement with bioprosthesis, mitral and tricuspid repair, closure of ventricular septal defect, bilateral MAZE and volume reduction of both atria. After a very complicated postoperative course with multiorgan failure he recovered and was discharged home 2 months after operation. The NYHA class improved from IV before operation to II. Twenty months after this operation he experienced infective endocarditis with a leak on the aortic bioprosthesis. He was reoperated with reimplantation of a new aortic bioprosthesis and with a very complicated postoperative course. Sildenafil was added to his therapy due to the persistent severe pulmonary hypertension. He survived and was discharged home. The presented unique high-risk complex reoperation of a congenital heart disease with pulmonary hypertension can be performed only by a very experienced cardiosurgical and cardio-anesthesiological team with a high level of the early and late postoperative care.

Dětské kardiocentrum FN Motol Praha

Interní oddělení Nemocnice Na Homolce Praha

Kardiocentrum Nemocnice Na Homolce Praha

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